Publicado

2009-07-01

Pseudosindactilia en epidermólisis bullosa.

Pseudosyndactylia in bullous epidermolysis

Palabras clave:

epidermólisis ampollosa, sindactilia, epidermólisis ampollosa distrófica. (es)
epidermolysis bullosa, syndactyly, epidermolysis bullosa dystrophica. (en)

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Autores/as

  • Enrique Vergara Amador Profesor asociado, Unidad de Ortopedia, Facultad de Medicina, Universidad Nacional de Colombia, Bogotá.
  • Henry Solaque Facultad de Medicina, Universidad Nacional de Colombia, Bogotá.
La epidermólisis bullosa comprende un grupo de patologías que se caracterizan por la fragilidad de la piel, formación de ampollas, y en las formas distróficas, la formación de pseudosindactilias. Debido a lo exótico de la entidad y la dificultad para el enfoque de tratamiento, se presenta un caso clínico con desarrollo de pseudosindactilias, característico usualmente de las formas distróficas. Se describe en aspectos clínicos de la entidad, el tratamiento quirúrgico de las pseudosindactilias y de las contracturas en las manos, y se discuten aspectos de la enfermedad y de los tipos de tratamiento.
Bullous epidermolysis comprises a group of pathologies characterized by skin fragility, formation of bullae and, in its dystrophic forms, by the formation of pseudosyndactylias. Due to the exotic nature of the disease and the difficulty in focusing its treatment, a case is presented in which pseudosyndactylias developed, a usual characteristic of the dystrophic forms. This article addresses clinical aspects of bullous epidermolysis, surgical treatment of pseudosyndactilia and hand contractures and other aspects of the disease and its treatment.

Referencias

Devries DT, Johnson LB, Weiner M, Fine JD. Relative extent of skin involvement in inherited epidermolysis bullosa (EB): composite regional anatomic diagrams based on the findings of the National EB Registry, 1986 to 2002. J Am Acad Dermatol. 2004; 50: 572-581.

Horn HM, Tidman MJ. The clinical spectrum of dystrophic epidermolysis bullosa. Br J. Dermatol. 2002; 146: 267-274.

Solovan C, Ciolan M, Olariu L. The biomolecular and ultrastructural basis of epidermolysis bullosa. Acta Dermatovenerol Alp Panonica Adriat. 2005; 14: 127- 135.

Pai S, Marinkovich P. Epidermolysis Bullosa. New and emerging trends. Am J Clin Dermatol. 2002; 3: 371-380.

Uitto J, Eady R, Fine JD, Feder M, Dart J. The DEBRA Internacional Visioning/Consensus Meeting on Epidermolysis Bullosa: Summary and Recommendations. J Invest Dermatol. 2000; 114: 734-737.

Fine JD, Johnson LB, Weiner M, Stein A, Cash S, Deleoz J, et al. Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: Experience of the National Epidermolysis Bullosa Registry, 1986–2002. J Hand Surg (Br). 2005; 30: 14-22.

Ladd AL, Kibele A, Gibbons S. Surgical treatment and postoperative splinting of recessive dystrophic epidermolysis bullosa. J Hand Surg (Br). 1996; 21: 888-897.

Terrill PJ, Mayou BJ, Pemberton J. Experience in the surgical management of the hand in dystrophic epidermolysis bullosa. Br J Plast Surg. 1992; 45: 435- 442.

Vozdvizhensky SI, Albanova VI. Surgical treatment of contracture and syndactyly of children with epidermolysis bullosa. Br J Plast Surg. 1993; 46: 314-316.

Pai S, Marinkovich P. Epidermolysis Bullosa New and Emerging Trends. Am J Clin Dermatol. 2002; 3: 371-380.

Fivenson D, Scherschun L, Cohen L. Apligraf in the Treatment of Severe Mitten Deformity Associated with Recessive Dystrophic Epidermolysis Bullosa. Plast Rec Surg. 2003; 584-588.

Phillips J, Rockwell WB. Surgical treatment of recessive Dystrophic Epidermolysis Bullosain the hand: Use of tissue-engineered skin (Apligraf). Ann Plast Surg. 2003; 50:441-442.

Ladd AL, Kibele A, Gibbons S. Surgical treatment and postoperative splinting of recessive dystrophic epidermolysis bullosa. J Hand Surg (Br). 1996; 21: 888-897.

Terrill PJ, Mayou BJ, Pemberton J. Experience in the surgical management of the hand in dystrophic epidermolysis bullosa. Br J Plast Surg. 1992; 45: 435- 442. 15. Vozdvizhensky SI, Albanova VI. Surgical treatment of contracture and syndactyly of children with epidermolysis bullosa. Br J Plast Surg. 1993; 46: 314-316.

Cavallo A, Smith P. Surgical Treatment of dystrophic epidermolysis bullosa of the hand. Tech Hand Up Extrem Surg. 1998; 2: 184-195.

Pajardi G, Pivato G, Rafanelli G. Rehabilitation in recessive dystrophic epidermolysis bullosa Tech Hand Up Extrem Surg. 2001; 5: 173-177.

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Cómo citar

Vergara Amador, E., & Solaque, H. (2009). Pseudosindactilia en epidermólisis bullosa. Revista De La Facultad De Medicina, 57(3), 274-280. https://revistas.unal.edu.co/index.php/revfacmed/article/view/14385

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Derechos de autor 2009 Revista de la Facultad de Medicina

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Esta obra está bajo una licencia Creative Commons Reconocimiento 3.0 Unported.

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